Mansfield family, community battle rare kidney disorder

Michael Mancuso/The TimesRobbinsville athletic director Curtis Wyers, left and his wife Leslie, 2nd from right, at home with their children, twin 8-year-old boys, Drew, left and Logan and daughter Kylie, 6.
Michael Mancuso/The Times

MANSFIELD — Curtis and Leslie Wyers can spell nephronophthisis without looking it up, and that’s not good. Only about one in a million families are in the position of having to learn the word.

Those are the odds of a child in this country having the kidney disorder, which is caused by an inherited genetic mutation and eventually leads to kidney failure.

The Wyers know because two of their three children have the condition.

Eight-year-old Logan was diagnosed last summer and had a kidney transplant last fall. His 6-year-old sister Kylie currently has 50 percent kidney function. Unless there is a cure, she will need a transplant by the time she’s a teenager.

Logan received a kidney from Leslie, and six months after the transplant was performed at the Children’s Hospital of Philadelphia, mother and child are fine. But as Leslie obviously cannot donate again for Kylie, and Curtis’ blood type does not provide a match, they will likely have to depend on an unknown donor.

For now they are searching for alternate treatments and trying to live normal lives — except when that’s impossible.

“We live, really — and it sounds crazy — minute by minute. It can all change so quickly,” said Curtis, the athletic director at Robbinsville High School. Leslie is a learning consultant in the West Windsor-Plainsboro school district.

“One day she’s driving home from the hospital and he’s vomiting in the car, and by the time she gets him in the house he’s peeing blood and she has to turn around and take him right back to Children’s,” Curtis said.

The disease has made the children particularly susceptible to infection. Sanitizers are mounted in almost every room in their house.

“It’s changed our lives,” their father said. “We have to be very cognizant about the slightest germs. We don’t have to live in a bubble by no means, but we have to be very aware of flus going around and viruses.”

They have started a website, www.nephhope.org, as they seek help for Kylie and the larger community of those affected nephronophthisis.

“Our mission is to further research and eventually find a cure, and also to bring families together who are afflicted. It will be a forum for us,” Leslie said.

“We’re not the type of family who sits on our heels,” Curtis said. “We don’t wait for people. We go out and do things ourselves.”

LOOKING FOR THE SPARK PLUG

On a recent afternoon at home, all was routine. Logan played with Kylie and with his twin brother Drew, who has no kidney problems. The Mets game was on TV, stuffed animals remained piled in a corner chair and next to the couch was a life-size cardboard cutout of Justin Bieber.

“That’s Kylie’s,’’ Drew was quick to point out.

Outside their pet horse and pony grazed not far from a swing set and trampoline cage.

In children who have nephronophthisis, cysts form in the kidney, the organ malfunctions and overall development is affected. Logan’s twin Drew, who has no kidney problems, is 35 pounds heavier than his brother at 100 pounds, and 8 inches taller at 4 feet, 8 inches.

“They call this the silent killer because you can go so long without knowing your kidneys are shutting down,’’ Leslie said. “Not so much shutting down as they aren’t cleaning toxins out of the body. So you get bone disease, heart disease, high blood pressure — secondary issues that cause a lot of the damage. So we’re obviously monitoring Kylie closely.’’

Yet the effect of successful transplants can be dramatic. Logan has named his new kidney Sparky, his mother having said the transplant was like getting a spark plug.
“In a really positive way, there was a kid we really didn’t know was sick, but now he’s like who Logan is supposed to be,” she said.

Logan has grown an inch over the past six months and added 20 pounds, his parents said. He was able to play in his school team’s final basketball game — he made a couple of three-pointers — is playing baseball. This summer will attend a Phillies baseball camp in Moorestown.

He needs to wear a kidney belt for activities, but aside from maybe not riding bumper cars at amusement parks he’s good to go.

Leslie and Curtis said they are frustrated by the lack of information about alternative treatments that might similarly help their daughter.

“I feel like something is going to come through for Kylie, I just believe, if we work really hard,” Leslie said. “We just pray.’’

Through their new website they will be looking for corporate donations, but more importantly for a pharmaceutical company that would run clinical trials of drugs that might slow the progress of the disease.

Anything to help their little girl as she endures the disease and repeated hospital visits.

“Logan’s kidney function was less than 10 percent when we had the procedure,” Leslie said. “With Kylie, it’s like, how do you sit by and watch a 6-year-old (suffer)? We asked the staff, what can we do?”

CHALLENGE AND HOPE

Sadly, the only answer to their questions was to search the internet. What they found every time they typed in the word nephronophthisis was a doctor named Friedhelm Hildebrant, a professor of pediatric and human genetics at the University of Michigan. The foremost authority on the disease — he actually discovered the gene — Hildebrant responded to Leslie’s email within two days.

“I explained our situation to him and told him I read articles where there were clinical trials with medications in mice that were working,” said Leslie. “I had asked why I couldn’t find a nephronophthisis support group. He said one doesn’t exist, but it would be a really good idea to start one, to create a fundraising site for researchers.’’

Curtis reached out to students at Robbinsville High and they created the web site, complete with logo. An attorney friend, the husband of a colleague of Leslie’s, handled the paperwork.

Through sharing their story, they also hope to draw more attention to kidney donation and, in Leslie’s words, to “demystify the donation process so that more people will be encouraged to become a living donor.”

Living with nephronophthisis has given the Wyers a different perspective on their lives and their community.

“This has all made us appreciate some things that maybe we overlooked or didn’t appreciate,’’Curtis said. “It really grounds you. We’ve become more religious.’’

“I can tell you for sure there is a God,’’ Leslie said. “There is a being, that, when you’re at your lowest, it pulls you back up. If you believe.’’

The neighborhood has rallied around the family, to the point where people came out in droves in a parade atmosphere when Logan returned home from the transplant in November.

“The community and the school districts, so many people have stepped up,’’ he said. “We’re very lucky with our jobs and the support we’ve received. People in this situation should know that there’s hope, as corny as that sounds. It will be challenging, but there is hope. And survival.”